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焦谷氨酸所致代谢性酸中毒

Metabolic Acidosis Due To Pyroglutamic Acid.

作者信息

Serpa Maria João, Falcão Luís, Franco Susana, Repolho Diana, Ferreira Nuno Ribeiro

机构信息

Internal Medicine Department, Hospital Beatriz Ângelo, Loures, Portugal.

Nephrology Department, Hospital Beatriz Ângelo, Loures, Portugal.

出版信息

Eur J Case Rep Intern Med. 2018 Oct 24;5(10):000949. doi: 10.12890/2018_000949. eCollection 2018.

Abstract

UNLABELLED

Metabolic acidosis is a common clinical disturbance due to increased plasma acidity caused by a primary decrease in serum HCO3- concentration. It is classified as normal or high anion gap metabolic acidosis. High anion gap metabolic acidosis can result from either a decrease in unmeasured cations (K, Ca2, Mg2) or an increase in unmeasured anions (PO , albumin). However, other anions such as lactic acid or keto acids may cause this acid-base disorder. It can also result from renal failure and intoxication (salicylate, methanol, ethylene glycol), or more rarely, from massive rhabdomyolysis and pyroglutamic (5-oxoproline) acidemia. Acidemia due to pyroglutamic acid should be considered when no other aetiology is found. High anion gap metabolic acidosis is diagnosed in children with inherited defects in enzymes of the γ-glutamyl cycle. In adults, this disorder from pyroglutamic acid has been described in association with chronic acetaminophen misuse. We report a case of pyroglutamic acidosis in a woman with acute misuse of acetaminophen concurrent with chronic use.

LEARNING POINT

Paracetamol is an easily available drug with potentially harmful consequences.Accumulation of pyroglutamic acid (5-oxoproline) may be a cause of high anion gap metabolic acidosis.Reporting cases of 5-oxoprolinemia acidosis contributes to better understanding of the condition.

摘要

未标注

代谢性酸中毒是一种常见的临床紊乱,由于血清HCO3-浓度原发性降低导致血浆酸度增加所致。它分为正常阴离子间隙或高阴离子间隙代谢性酸中毒。高阴离子间隙代谢性酸中毒可由未测定阳离子(K、Ca2、Mg2)减少或未测定阴离子(PO4、白蛋白)增加引起。然而,其他阴离子如乳酸或酮酸也可能导致这种酸碱紊乱。它也可由肾衰竭和中毒(水杨酸盐、甲醇、乙二醇)引起,或更罕见地由大量横纹肌溶解和焦谷氨酸(5-氧代脯氨酸)酸血症引起。当未发现其他病因时,应考虑焦谷氨酸引起的酸血症。在γ-谷氨酰循环酶存在遗传性缺陷的儿童中可诊断出高阴离子间隙代谢性酸中毒。在成人中,这种由焦谷氨酸引起的疾病已被描述与长期滥用对乙酰氨基酚有关。我们报告一例急性滥用对乙酰氨基酚并伴有长期使用的女性发生焦谷氨酸酸中毒的病例。

学习要点

对乙酰氨基酚是一种容易获得的药物,可能会产生有害后果。焦谷氨酸(5-氧代脯氨酸)的蓄积可能是高阴离子间隙代谢性酸中毒的一个原因。报告5-氧代脯氨酸血症酸中毒病例有助于更好地了解这种情况。

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